Chimerism and tolerance in a recipient of a deceased-donor liver transplant.

Complete hematopoietic chimerism and tolerance of a liver allograft from a deceased male donor developed in a 9-year-old girl, with no evidence of graft-versus-host disease 17 months after transplantation. The tolerance was preceded by a period of severe hemolysis, reflecting partial chimerism that was refractory to standard therapies. The hemolysis resolved after the gradual withdrawal of all immunosuppressive therapy.

Complex phenotypes in the haemoglobinopathies: recommendations on screening and DNA testing.

This document considers a number of scenarios involving complex haemoglobinopathies and provides 28 recommendations at both the clinical and laboratory levels on how these should be managed.

Recombinant activated factor VII in paediatric cardiac surgery.

Objective: To review the use of recombinant activated factor VII in paediatric cardiac surgery.

Design: Retrospective chart review.

Setting: Paediatric intensive care unit in a stand-alone university-affiliated children's hospital.

Intermittent 20q- and consistent i(7q) in a patient with Shwachman-Diamond syndrome.

Shwachman Diamond syndrome (SDS) is a genetic disorder characterized by pancreatic hypoplasia, recurrent infection and bone marrow dysfunction. Some cases have an abnormality of chromosome 7, such as isochromosome 7q (i(7q)), which may be associated with the development of leukemia. We present a boy who was diagnosed with SDS at 19 months of age.